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1.
Medicine (Baltimore) ; 98(31): e16571, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31374024

RESUMO

RATIONALE: IgG4-related disease (IgG4-RD) is a systemic autoimmune disease and mixed cryoglobulinemia may be caused by autoimmune diseases. However, so far only 1 case of IgG4-RD complicated with mixed cryoglobulinemia is reported. Our case further confirms the close relationship between these 2 diseases. PATIENT CONCERNS: A 55-year-old female was admitted because of dry mouth and teeth falling off. DIAGNOSES: The patient was diagnosed as IgG4-related sialadenitis (IgG4-RS) complicated with type III mixed cryoglobulinemia. IgG4-RS was confirmed by elevated serum IgG4 levels and diffuse IgG4 plasmocyte infiltration and storiform fibrosis in the interstitium of labial gland. Type III mixed cryoglobulinemia was confirmed by positive serum cryoglobulins and no monoclonal immunoglobulin in serum and urine. INTERVENTIONS AND OUTCOMES: After treatment with prednisone and cyclophosphamide, serum cryoglobulins rapidly turned negative with the remission of IgG4-RS. LESSONS: Type III mixed cryoglobulinemia can be caused by IgG4-RS, and the underlying mechanisms need to be further explored.


Assuntos
Crioglobulinemia/complicações , Doença Relacionada a Imunoglobulina G4/complicações , Sialadenite/complicações , Crioglobulinemia/tratamento farmacológico , Feminino , Humanos , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Imunossupressores/uso terapêutico , Pessoa de Meia-Idade , Sialadenite/tratamento farmacológico
2.
Medicine (Baltimore) ; 98(28): e16382, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31305439

RESUMO

RATIONALE: Type I monoclonal cryoglobulinemia is usually associated with lymphoproliferative disorders, such as monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukemia (CLL) and lymphoplasmocytic lymphoma (LPL). Clinical symptoms related to Type I cryoglobulin (CG) isotype often include skin, neurological and renal manifestations. PATIENT CONCERNS: A 42-year-old woman who initially presented urticaria, palpable purpura in both her upper extremities and legs, eosinophilia and Raynaud phenomenon. Skin biopsy revealed eosinophil infiltration. Monoclonal immunoglobulin (Ig) G-κprotein was detected and CG was also positive. DIAGNOSES: The patient was finally diagnosed as MGUS related Type I CG. INTERVENTIONS: Cyclophosphamide-dexamethasone-thalidomide (CDT) therapy was initiated. OUTCOMES: The treatment relieved the skin symptoms efficiently. LESSONS: To our knowledge, this is a rare case of Type I cryoglobulinemic vasulitis with eosinophilia complicated by MGUS, and the effective treatment of cyclophosphamide combined with thalidomide and prednisone may provide a new therapeutic option for cryoglobulinemic vasulitis.


Assuntos
Crioglobulinemia/complicações , Eosinofilia/complicações , Gamopatia Monoclonal de Significância Indeterminada/complicações , Vasculite/complicações , Adulto , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Diagnóstico Diferencial , Eosinofilia/diagnóstico , Eosinofilia/tratamento farmacológico , Feminino , Humanos , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/tratamento farmacológico , Vasculite/diagnóstico , Vasculite/tratamento farmacológico
3.
BMC Infect Dis ; 19(1): 636, 2019 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-31315560

RESUMO

BACKGROUND: We describe a case of severe Guillain-Barre syndrome (GBS) associated with chronic active hepatitis C and mixed cryoglobulinemia (MC). To our knowledge, this association between GBS and hepatitis C virus (HCV) infection has been rarely reported. CASE PRESENTATION: A 56-year-old man developed symmetrical muscle weakness in all extremities, areflexia and sensorial disorder followed by acute respiratory failure associated with chronic active hepatitis C, which was confirmed by the presence of anti-HCV antibodies in the serum and persistence of HCV RNA viral load for more than 6 months. Chronic hepatitis C was further complicated by type 3 MC. Electromyography showed peripheral nerve injury (mainly in axon). A severe acute motor sensory axonal neuropathy (AMSAN) was diagnosed. After treatment with intravenous immunoglobulin and plasma exchange followed by antiviral therapy by direct-acting antiviral agent, patient showed progressive recovery and was transferred 3 months after his first admission to a rehabilitation center. CONCLUSIONS: Our case reported a severe GBS associated with HCV infection and MC. EMG classified for the first time the subtype of GBS (severe AMSAN) correlated with severe clinical form. HCV infection should be screened in high-risk patients to prevent silent progression of the chronic hepatitis C and its potentially severe extra-hepatic manifestations.


Assuntos
Crioglobulinemia/etiologia , Síndrome de Guillain-Barré/etiologia , Hepatite C Crônica/etiologia , Antivirais/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Hepatite C Crônica/complicações , Hepatite C Crônica/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Pessoa de Meia-Idade , Síndrome do Desconforto Respiratório do Adulto/tratamento farmacológico , Síndrome do Desconforto Respiratório do Adulto/etiologia
4.
Saudi J Kidney Dis Transpl ; 30(3): 663-669, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31249231

RESUMO

Cryoglobulinemia is a rare cause of kidney disease that occurs in patients with various diseases. Renal involvement often occurs after appearance of various clinical manifestations dominated by purpura and neuropathy. The aim of this study is to describe clinical, biological, and pathological characteristics of cryoglobulinemic glomerulonephritis (GN), as well as treatment and outcome. This is a retrospective study including all patients with positive cryoglobulin test and biopsy-proven GN secondary to cryoglobulinemia. Fourteen patients with cryoglobulinemic GN were collected. Their mean age was 46.92 ± 15.82 years with male predominance (64.28%). Weight loss, fever (71.42%), and purpuric rash (57.14%) were the main extrarenal manifestations. Eight patients presented with nephrotic syndrome (NS), associated with renal impairment in three patients. Four patients had rapidly progressive GN and two patients had acute kidney injury. Renal biopsy, performed in all patients, revealed membranoproliferative GN with glomerular thrombi in all patients. Crescents and necrotizing vasculitis were present in four patients. Hepatitis C virus (HCV) infection was the most common etiology. Antivirals and steroids or other immunosuppressive agents were used in most of the patients. During follow-up, complete response was observed in three patients and partial response was observed in four patients. Five patients had no response with renal injury requiring hemodialysis. NS with hematuria and renal insufficiency were the main clinical manifestations of cryoglobulinemic GN. In our study, HCV infection dominated the etiologies, although not well described earlier. A half of our patients had poor outcome even after antiviral and immunosuppressive therapy.


Assuntos
Lesão Renal Aguda/etiologia , Crioglobulinemia/etiologia , Glomerulonefrite Membranoproliferativa/etiologia , Hepatite C/complicações , Departamentos Hospitalares , Nefrologia , Síndrome Nefrótica/etiologia , Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/imunologia , Lesão Renal Aguda/terapia , Adulto , Antivirais/uso terapêutico , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/imunologia , Feminino , Glomerulonefrite Membranoproliferativa/diagnóstico , Glomerulonefrite Membranoproliferativa/imunologia , Glomerulonefrite Membranoproliferativa/terapia , Hepatite C/diagnóstico , Hepatite C/tratamento farmacológico , Hepatite C/imunologia , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Marrocos , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/imunologia , Síndrome Nefrótica/terapia , Diálise Renal , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
5.
Reumatol. clín. (Barc.) ; 15(3): 173-178, mayo-jun. 2019. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-184371

RESUMO

Objetivo: Revisar la eficacia y seguridad del rituximab en neuropatía vasculítica (NV). Métodos: Se realizó una búsqueda en la literatura de Medline y Embase hasta 2017. Los términos incluidos guardaron relación con «vasculitis», «neuropatía vasculítica» y «rituximab». Dicha búsqueda fue realizada por 2 revisores. El resultado principal fue la eficacia del rituximab. Resultados: Tras seleccionar inicialmente 702 artículos, 5 de ellos permanecieron con un nivel de evidencia de entre 1+ y 3, y un grado de recomendación variable. En el único ensayo clínico incluido, el rituximab fue superior a la terapia convencional para vasculitis crioglobulinémica, mostrando NV un incremento en la tasa de retención farmacológica (64,3 vs. 3,5%; p<0,001) y una menor tasa de efectos adversos graves (0,12 vs. 0,48). Los estudios de cohortes de pacientes con vasculitis crioglobulinémica incluyeron una mejora probada y una remisión completa/parcial de NV. En una serie de 5 casos de EGPA refractaria con NV, el 60% y el 20% de los pacientes lograron una remisión completa y parcial, respectivamente. Conclusiones: Rituximab parece ser un tratamiento eficaz y seguro para NV, en el contexto de vasculitis crioglobulinémica. Se carece de evidencia sobre la eficacia específica de NV en el contexto de otras vasculitis


Objective: To review the efficacy and safety of rituximab in vasculitic neuropathy (VN). Methods: A literature search was performed on Medline and Embase up until 2017. It included terms related to "vasculitis","vasculitic neuropathy" and "Rituximab". Research was carried out by two reviewers. The main outcome was rituximab efficacy. Results: Of an initial selection of 702 articles, 5 remained with a level of evidence between 1+ and 3 and variable recommendation degree. In the only clinical trial included, rituximab was superior to conventional therapy for cryoglobulinemic vasculitis with VN showing an increase in drug retention rate (64.3% vs. 3.5%; P<.001)and with a lower rate of serious adverse effects (.12 vs. .48). Cohort studies of patients with cryoglobulinemic vasculitis showed improvement and complete/partial remission of VN. In a series of 5 cases of refractory EGPA suffering from VN, 60% and 20% of patients achieved complete and partial remission respectively. Conclusions: Rituximab seems an effective and safe treatment for VN in the context of cryoglobulinemic vasculitis. Evidence for specific efficacy in VN in the context of other types of vasculitis is lacking


Assuntos
Humanos , Rituximab/uso terapêutico , Vasculite do Sistema Nervoso Central/tratamento farmacológico , Crioglobulinemia/tratamento farmacológico , Resultado do Tratamento , Segurança do Paciente
6.
BMJ Case Rep ; 12(5)2019 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-31138600

RESUMO

Cryofibrinogenaemia is a rare haematological disorder characterised by cold temperature-induced precipitation of plasma proteins causing small-vessel occlusive vascular disorder with a hallmark of skin ulceration. It remains an underdiagnosed entity because of a lack of diagnostic criteria. Cryoglobulinaemia vasculitis is a small-vessel vasculitis involving the skin, the joints, the peripheral nerve system and the kidneys. Its association with cryofibrinogenaemia causes more severe phenotype with poor prognosis. We describe the case of a 59-year-old woman presenting with cold-induced extensive bilateral foot gangrene due to coexisting cryofibrinogenaemia and cryoglobulinaemic vasculitis that required bilateral amputation and rituximab perfusions as maintenance therapy.


Assuntos
Crioglobulinemia/diagnóstico , Pé/irrigação sanguínea , Gangrena/etiologia , Amputação/métodos , Antirreumáticos/uso terapêutico , Crioglobulinemia/complicações , Crioglobulinemia/tratamento farmacológico , Diagnóstico Diferencial , Feminino , Pé/patologia , Gangrena/patologia , Gangrena/cirurgia , Humanos , Pessoa de Meia-Idade , Doenças Raras , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Resultado do Tratamento , Vasculite/complicações
8.
Medicine (Baltimore) ; 98(4): e14201, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30681592

RESUMO

RATIONALE: Concomitant cryoglobulinemic vasculitis and cold agglutinin disease (CAD) is an extremely uncommon clinical scenario. The role of bortezomib in the treatment of cryoglobulinemic vasculitis needs further investigation. PATIENT CONCERNS: A 72-year-old Chinese woman presented with a 25-year history of cyanosis of the extremities after cold exposure, which worsened and was accompanied with purpuric skin lesions and proteinuria in recent years. Laboratory data demonstrated hemolysis. Cold agglutinin and cryoglobulin tests were positive. There was no evidence for malignancies after blood, image, and pathologic tests. DIAGNOSES: Concomitant cryoglobulinemic vasculitis and CAD. INTERVENTIONS: The patient was treated with bortezomib-based regimen, including bortezomib, cyclophosphamide, and dexamethasone. OUTCOMES: The patient responded well to the treatment. Both symptoms and laboratory tests significantly improved. The patient's condition was in a state of sustained remission in the 6-month follow-up. LESSONS: This rare case promotes further understanding of these 2 diseases and suggests that bortezomib is a promising treatment in type I cryoglobulinemic vasculitis.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Bortezomib/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Vasculite/tratamento farmacológico , Idoso , Anemia Hemolítica Autoimune/complicações , Crioglobulinemia/complicações , Ciclofosfamida/uso terapêutico , Dexametasona/uso terapêutico , Feminino , Humanos , Resultado do Tratamento , Vasculite/complicações
9.
Reumatol Clin ; 15(3): 173-178, 2019.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30691946

RESUMO

OBJECTIVE: To review the efficacy and safety of rituximab in vasculitic neuropathy (VN) METHODS: A literature search was performed on Medline and Embase up until 2017. It included terms related to "vasculitis","vasculitic neuropathy" and "Rituximab". Research was carried out by two reviewers. The main outcome was rituximab efficacy. RESULTS: Of an initial selection of 702 articles, 5 remained with a level of evidence between 1+ and 3 and variable recommendation degree. In the only clinical trial included, rituximab was superior to conventional therapy for cryoglobulinemic vasculitis with VN showing an increase in drug retention rate (64.3% vs. 3.5%; P<.001)and with a lower rate of serious adverse effects (.12 vs. .48). Cohort studies of patients with cryoglobulinemic vasculitis showed improvement and complete/partial remission of VN. In a series of 5 cases of refractory EGPA suffering from VN, 60% and 20% of patients achieved complete and partial remission respectively. CONCLUSIONS: Rituximab seems an effective and safe treatment for VN in the context of cryoglobulinemic vasculitis. Evidence for specific efficacy in VN in the context of other types of vasculitis is lacking.


Assuntos
Crioglobulinemia/tratamento farmacológico , Imunossupressores/uso terapêutico , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Rituximab/uso terapêutico , Vasculite/tratamento farmacológico , Ensaios Clínicos como Assunto , Estudos de Coortes , Crioglobulinemia/complicações , Humanos , Imunossupressores/efeitos adversos , Metanálise como Assunto , Estudos Multicêntricos como Assunto , Estudos Observacionais como Assunto , Doenças do Sistema Nervoso Periférico/etiologia , Rituximab/efeitos adversos , Vasculite/complicações
10.
BMJ Case Rep ; 12(1)2019 Jan 04.
Artigo em Inglês | MEDLINE | ID: mdl-30612104

RESUMO

We report an interesting case of hepatitis C virus-negative type II cryoglobulinaemic vasculitis (CV) in a patient with a background history of systemic lupus erythematosus. The type II CV became less responsive to traditional treatments over time and culminated in an intensive care unit admission with critical multiorgan failure. A detailed flow cytometric evaluation of the bone marrow proved to be helpful in treatment. It demonstrated that bortezomib was a viable alternative treatment option for the type II CV. The patient received bortezomib and has made a full and durable recovery.


Assuntos
Bortezomib/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Lúpus Eritematoso Sistêmico/complicações , Vasculite/tratamento farmacológico , Administração Intravenosa , Assistência ao Convalescente , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Medula Óssea/imunologia , Bortezomib/administração & dosagem , Crioglobulinemia/diagnóstico , Feminino , Citometria de Fluxo/métodos , Hepatite C/complicações , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/complicações , Resultado do Tratamento , Vasculite/diagnóstico , Vasculite/imunologia
11.
Eur J Gastroenterol Hepatol ; 30(10): 1208-1215, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30138160

RESUMO

OBJECTIVES: Hepatitis C virus (HCV) is the major cause of cryoglobulinemia. Direct-acting antivirals (DAAs) have markedly changed the therapeutic outcomes in the treatment of patients with HCV. We evaluate the efficacy, safety, immunological, and clinical response of different DAA regimens in HCV-cryoglobulinemia. PATIENTS AND METHODS: Ninety-three cryoglobulinemic patients, divided into symptomatic [symptomatic cryoglobulinemic patients (SCP; n=35)] and asymptomatic [nonsymptomatic cryoglobulinemic patients (NSCP; n=60)], underwent DAAs. Eighty-nine comparable noncryoglobulinemic patients were selected as a control group. We evaluated the sustained virological response (SVR), the adverse effects, and the immune and symptomatic response. RESULTS: Percentages of patients who achieved SVR and experienced adverse effects were not statistically different between the three groups (100, 95, 93.3% and 57.1, 53.3, 48.3%). In 68.5% of SCP and in 76.7% of NSCP, cryoglobulins disappeared at SVR. No risk factor was associated with the persistence of cryoglobulins. An increase was observed both in C4 (P=0.002; P=0.018) and in C3 (P=0.0037; P=0.031) in SCP and NSCP. About 70% of symptomatic patients showed a complete or partial symptomatic remission: persistence of symptoms is correlated to the type of clinical picture. CONCLUSION: DAA regimens are safe and effective in patients with HCV-cryoglobulinemia. The achievement of SVR is necessary, but not sufficient, to achieve a complete immunological and clinical response.


Assuntos
Antivirais/efeitos adversos , Crioglobulinemia/tratamento farmacológico , Crioglobulinas/metabolismo , Hepatite C Crônica/tratamento farmacológico , Idoso , Antivirais/uso terapêutico , Complemento C3/metabolismo , Complemento C4/metabolismo , Crioglobulinemia/sangue , Crioglobulinemia/imunologia , Crioglobulinemia/virologia , Quimioterapia Combinada/efeitos adversos , Feminino , Hepatite C Crônica/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Resposta Viral Sustentada
12.
EBioMedicine ; 35: 106-113, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30139628

RESUMO

Mixed Cryoglobulinemic Vasculitis (MCV) is a prominent extra-hepatic manifestation of Hepatitis C virus (HCV) infection. HCV has been reported to cause B-cell disorders and genomic instability. Here, we investigated B-cell activation and genome stability in HCV-MCV patients receiving the direct antiviral agent, Sofosbuvir, at multiple centers in Egypt. Clinical manifestations in HCV-MCV patients were improved at the end of treatment (EOT), such as purpura (100%), articular manifestations (75%) and neuropathy (68%). Eighteen patients (56%) showed vasculitis relapse after EOT. BAFF and APRIL were higher at EOT and continued to increase one year following treatment onset. Chromosomal breaks were elevated at EOT compared to baseline levels and were sustained at 3 and 6 months post treatment. We report increased expression of DNA genome stability transcripts such as topoisomerase 1 and TDP1 in HCV-MCV patients after treatment, which continued to increase at 12 months from treatment onset. This data suggest that B-cell activation and DNA damage are important determinants of HCV-MCV treatment outcomes.


Assuntos
Antivirais/farmacologia , Instabilidade Genômica/efeitos dos fármacos , Hepacivirus/efeitos dos fármacos , Antivirais/uso terapêutico , Fator Ativador de Células B/metabolismo , Linfócitos B/efeitos dos fármacos , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/patologia , Crioglobulinemia/virologia , Crioglobulinas/metabolismo , Dano ao DNA , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Sofosbuvir/farmacologia , Sofosbuvir/uso terapêutico , Membro 13 da Superfamília de Ligantes de Fatores de Necrose Tumoral/metabolismo , Vasculite/tratamento farmacológico , Vasculite/patologia , Vasculite/virologia
13.
BMC Nephrol ; 19(1): 170, 2018 07 09.
Artigo em Inglês | MEDLINE | ID: mdl-29986657

RESUMO

BACKGROUND: Recurrence of glomerulonephritis is an important risk factor for renal graft dysfunction. Cryoglobulinemia is known as a relatively rare cause of renal failure, and doctors are usually hesitant to perform transplantation on a recipient with cryoglobulinemia because of the risk for graft loss. We present a case of renal transplantation on a patient with organ manifestations of type II cryoglobulinemia. CASE PRESENTATION: At the age of 44 years, the patient developed acute kidney injury and purpura on the lower extremities with type II cryoglobulinemia after interferon therapy for hepatitis C virus. Cryoglobulinemic glomerulonephritis was suspected; however, despite immunosuppressive therapy combined with plasmapheresis, she eventually needed hemodialysis treatment. She was referred to us at the age of 49 years for renal transplantation. Cryocrit was 14% and the organ manifestations persisted, including the lower extremity purpura and neurologic symptoms. After monitoring and confirming sufficient suppression of cryoglobulin concentration by immunosuppressive treatment with prednisolone, cyclophosphamide, and rituximab combined with plasmapheresis, the operation was performed. After transplantation, the cryoglobulin concentration was continuously monitored, and plasmapheresis and rituximab infusion were performed as appropriate. Her graft function has remained stable for 2 years and 6 months. CONCLUSION: Our case suggested that a patient with cryoglobulinemia and persistent organ manifestations can receive a renal graft if the cryoglobulin concentration is sufficiently controlled by pretransplant treatment.


Assuntos
Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/cirurgia , Crioglobulinemia/diagnóstico , Crioglobulinemia/cirurgia , Transplante de Rim/tendências , Doadores Vivos , Lesão Renal Aguda/tratamento farmacológico , Idoso , Crioglobulinemia/tratamento farmacológico , Feminino , Humanos , Imunossupressores/uso terapêutico , Transplante de Rim/métodos , Pessoa de Meia-Idade , Resultado do Tratamento
14.
Aliment Pharmacol Ther ; 48(4): 440-450, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29952013

RESUMO

BACKGROUND: Cryoglobulinaemic vasculitis (CV) is a lymphoproliferative disorder related to hepatitis C virus (HCV) infection; anti-viral therapy is the first therapeutic option. CV can be incapacitating, compromising the patients' quality of life (QoL). In a controlled study, interferon-based therapy was associated with a lower virological response in vasculitic patients than in patients without vasculitis. Limited, uncontrolled data on direct-acting anti-virals are available. AIM: To evaluate safety, clinical efficacy, virological response and the impact of interferon-free treatment on QoL in HCV patients with and without mixed cryoglobulinaemia (MC). METHODS: We prospectively studied HCV patients with cryoglobulinaemia (with vasculitis-CV- and without vasculitis-MC-) and without cryoglobulinaemia (controls), treated with direct-acting anti-virals. Hepato-virological parameters, CV clinical response and impact on QoL were assessed. RESULTS: One hundred and eighty-two HCV patients were recruited (85 with CV, 54 with MC and 43 controls). A sustained virological response at 12 weeks (SVR12) was achieved in 166 (91.2%) patients (77/85 CV, 48/54 MC, 41/43 controls). In CV SVR patients, cryocrit levels progressively decreased and clinical response progressively improved, reaching 96.7%, 24 weeks after treatment. QoL, baseline physical and mental component summaries were lower in the CV group compared to the other groups (P < 0.05). Scores improved in all groups, and significantly in CV patients after SVR. CONCLUSIONS: No significant differences in SVR rates were recorded between cryoglobulinaemic patients and controls and a high clinical and immunological efficacy was confirmed in CV, supporting the role of interferon-free therapy as the first therapeutic option. Interestingly, CV patients had worse baseline QoL than other HCV-positive groups and interferon-free therapy was effective in significantly increasing QoL, suggesting the important role of direct-acting anti-viral-based therapy in improving CV's individual and social burden.


Assuntos
Antivirais/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/virologia , Hepatite C Crônica/complicações , Hepatite C Crônica/tratamento farmacológico , Adulto , Idoso , Feminino , Hepacivirus/fisiologia , Humanos , Imunoterapia , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Resposta Viral Sustentada , Resultado do Tratamento
16.
Clin Exp Rheumatol ; 36 Suppl 111(2): 12-32, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29799395
18.
CEN Case Rep ; 7(2): 211-216, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29671215

RESUMO

Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren's syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis. Before kidney biopsy, tubulointerstitial nephritis probably due to Sjögren's syndrome was suspected because of persistent hematuria without significant proteinuria and developing mild renal dysfunction over 6 months. The developing renal dysfunction associated with isolated hematuria is uncommon in glomerular diseases. Kidney biopsy, however, revealed established membranoproliferative glomerulonephritis with subendothelial deposits consisting of tubular structures with IgM, IgG, and C3 staining. Corticosteroids plus mycophenolate mofetil therapy successfully normalized renal function. Physician should not overlook cryoglobulinemic glomerulonephritis, which is potentially poor prognosis, even if urinalysis shows only persistent isolated hematuria in patients with autoimmune diseases.


Assuntos
Crioglobulinemia/tratamento farmacológico , Glomerulonefrite Membranoproliferativa/tratamento farmacológico , Glomerulonefrite/tratamento farmacológico , Hematúria/diagnóstico , Rim/fisiopatologia , Corticosteroides/uso terapêutico , Idoso , Antibióticos Antineoplásicos/uso terapêutico , Doenças Autoimunes/complicações , Crioglobulinemia/complicações , Crioglobulinemia/patologia , Feminino , Glomerulonefrite/complicações , Glomerulonefrite/patologia , Glomerulonefrite Membranoproliferativa/complicações , Glomerulonefrite Membranoproliferativa/patologia , Doença de Hashimoto/complicações , Hematúria/etiologia , Hepatite Autoimune/complicações , Humanos , Rim/imunologia , Rim/patologia , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Ácido Micofenólico/administração & dosagem , Ácido Micofenólico/uso terapêutico , Nefrite Intersticial/complicações , Pancitopenia/complicações , Pancitopenia/imunologia , Insuficiência Renal/complicações , Síndrome de Sjogren/complicações , Resultado do Tratamento
19.
Intern Med ; 57(17): 2511-2515, 2018 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-29607961

RESUMO

We report the case of a 75-year-old woman who was found to have hepatitis C virus (HCV) infection in 1987. Before treatment in 2016, she was found to have mixed cryoglobulinemia (MC). Direct-acting antiviral (DAA) treatment produced a sustained virological response 12 (SVR12). She noticed gradual muscle weakness in 2015 and the gradual development of dysarthria and dysphagia in 2017. We performed a muscle biopsy that showed inclusion body myositis (IBM). To the best of our knowledge, this is first case of a patient with HCV infection, MC, and IBM, in which MC and IBM did not improve after an SVR12 was obtained by DAA treatment.


Assuntos
Antivirais/uso terapêutico , Crioglobulinemia/complicações , Hepatite C Crônica/complicações , Hepatite C Crônica/tratamento farmacológico , Miosite de Corpos de Inclusão/complicações , Idoso , Anilidas/uso terapêutico , Carbamatos/uso terapêutico , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Feminino , Hepatite C Crônica/diagnóstico , Humanos , Compostos Macrocíclicos/uso terapêutico , Miosite de Corpos de Inclusão/diagnóstico , Miosite de Corpos de Inclusão/tratamento farmacológico , Ritonavir/uso terapêutico , Resposta Viral Sustentada
20.
Clin Exp Rheumatol ; 36(3): 490-493, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29533748

RESUMO

OBJECTIVES: Guidelines for preventing Pneumocystis pneumonia (PCP) in HIV patients are based on CD4 below 200/mm3. Such cut-off value is suggested to guide prophylaxis in non-HIV conditions (NHIV) especially in autoimmune and inflammatory diseases (AD). We aimed to determine if CD4 could be used to guide PCP prophylaxis in AD. METHODS: CD4 and lymphocyte-count were retrospectively studied in patients diagnosed with PCP between January 2013 and February 2016. RESULTS: 129 patients were included. The median CD4-count was 302/mm3 in AD, which was significantly higher than in HIV patients (19/mm3; p<0.0001). Fifty percent (n=10) of AD patients had CD4 counts greater than 300/mm3. CONCLUSIONS: Prophylaxis for PCP cannot rely solely on CD4-count in NHIV patients especially in AD.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Linfócitos T CD4-Positivos/imunologia , Infecções por HIV/terapia , Imunossupressores/efeitos adversos , Linfopenia/tratamento farmacológico , Pneumonia por Pneumocystis/prevenção & controle , Adulto , Idoso , Idoso de 80 Anos ou mais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/imunologia , Doenças Autoimunes/complicações , Doenças Autoimunes/imunologia , Contagem de Linfócito CD4 , Crioglobulinemia/complicações , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/imunologia , Dermatomiosite/complicações , Dermatomiosite/tratamento farmacológico , Dermatomiosite/imunologia , Gerenciamento Clínico , Feminino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/imunologia , Infecções por HIV/complicações , Hepatite Autoimune/complicações , Hepatite Autoimune/tratamento farmacológico , Hepatite Autoimune/imunologia , Humanos , Hospedeiro Imunocomprometido , Linfopenia/etiologia , Linfopenia/imunologia , Masculino , Pessoa de Meia-Idade , Neoplasias/complicações , Neoplasias/imunologia , Neoplasias/terapia , Transplante de Órgãos , Pneumonia por Pneumocystis/etiologia , Pneumonia por Pneumocystis/imunologia , Guias de Prática Clínica como Assunto , Estudos Retrospectivos
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